Background: Chronic myeloid leukemia (CML) is a rare disease entity in the pediatric age group constituting about 3-5% of childhood leukemia. The natural history and biologic characteristics of the disease are similar to adult CML. In addition, the response to therapy (tyrosine kinase inhibitor) is equally remarkable with no limiting side effect reported thus far. Case Report: We report a rare case of BCR-ABL positive CML in a 16 year old teenage girl. Conclusion: The aim of this study is to bring to the similarities in the molecular pathology and management between this very rare childhood leukemia and the adult-type.